Yoshida K
Department of Neurology, Hokkaido University School of Medicine, Sapporo, Japan.
Hokkaido Igaku Zasshi. 1997 Mar;72(2):163-80.
The modified Gomori trichrome stain of muscles can demonstrate ragged red fibers which are irregular in outline and display a thick and irregular red subsarcolemmal layer and intermyofibrillar red deposits. Typical ragged red fibers are often encountered in mitochondrial myopathy. On the other hand, we have noticed fibers outlined by a thin red subsarcolemmal layer. These fibers are smooth in outline. The sarcoplasm shows normal intermyofibrillar network. We defined these fibers as "smooth red fibers". To investigate the pathological significance of the smooth red fibers, we studied morphological differences between the smooth red fibers and ragged red fibers by light and electron microscopy and evaluated the occurrence and characteristics of the both abnormal muscle fibers in several neuromuscular diseases. Muscle specimens from 738 patients who were seen or consulted at the Department of Neurology, Hokkaido University, from January 1980 to October 1994 were examined. The smooth red fibers were classified into two types, type I and type II. Type I smooth red fibers were hypertrophied and showed a thin smooth red margin. Electron microscopy of the type I smooth red fibers showed no mitochondrial abnormality, being different from ragged red fibers which have abnormal mitochondria. Type I smooth red fibers were observed in chronic denervation process; they were specially frequent in Kugelberg-Welander syndrome. Hypertrophy of type I smooth red fibers were considered to be a compensative reaction in chronic denervation. Type II smooth red fibers were observed with or without ragged red fibers in mitochondrial myopathy. Type II smooth red fibers showed a thin smooth red margin, spreading red deposits from the margin into sarcoplasm. The fibers showed mitochondrial abnormality in electron microscopy. It could be posturated that type II smooth red fibers were transformed into ragged red fibers. The findings suggest 1) type I and type II smooth red fibers are different in origin, 2) type II smooth red fibers change to ragged red fibers, 3) type I and type II smooth red fibers are important markers of chronic denervation and mitochondrial myopathy, respectively. These two types of smooth red fibers are worthy of attention in muscle pathological observations.
改良的戈莫里三色肌染色可显示出边缘不规则的破碎红纤维,其肌膜下有一层粗大且不规则的红色层以及肌原纤维间红色沉积物。典型的破碎红纤维常见于线粒体肌病。另一方面,我们注意到一些纤维的肌膜下有一层薄的红色边缘。这些纤维边缘光滑。肌浆显示正常的肌原纤维网络。我们将这些纤维定义为“光滑红纤维”。为了研究光滑红纤维的病理意义,我们通过光镜和电镜研究了光滑红纤维与破碎红纤维之间的形态学差异,并评估了这两种异常肌纤维在几种神经肌肉疾病中的发生情况和特征。对1980年1月至1994年10月在北海道大学神经科就诊或咨询的738例患者的肌肉标本进行了检查。光滑红纤维分为I型和II型。I型光滑红纤维肥大,边缘薄且光滑。I型光滑红纤维的电镜检查显示线粒体无异常,这与有异常线粒体的破碎红纤维不同。I型光滑红纤维见于慢性失神经过程;在库格尔贝格 - 韦兰德综合征中尤为常见。I型光滑红纤维的肥大被认为是慢性失神经的一种代偿反应。II型光滑红纤维在伴有或不伴有破碎红纤维的线粒体肌病中均可观察到。II型光滑红纤维边缘薄且光滑,红色沉积物从边缘延伸至肌浆。电镜检查显示这些纤维有线粒体异常。可以推测II型光滑红纤维会转变为破碎红纤维。这些发现提示:1)I型和II型光滑红纤维起源不同;2)II型光滑红纤维会转变为破碎红纤维;3)I型和II型光滑红纤维分别是慢性失神经和线粒体肌病的重要标志物。这两种类型的光滑红纤维在肌肉病理观察中值得关注。